Abstract Library

#4353 Familial inactivating glucagon receptor mutation resulting in pancreatic neuroendocrine tumours with metastatic potential, somatic MEN1 mutations, and a heterozygous phenotype

Introduction: Homozygous pathogenic glucagon receptor (GCGR) mutations cause a syndrome with pancreatic glucagon cell hyperplasia and neoplasia (GCHN) associated with Mahvash disease. This is an exceptionally rare autosomal recessive hereditary pancreatic neuroendocrine tumour (panNET) syndrome, with approximately ten cases documented in the literature.

Conference:

Presenting Author: Kuiper J

Authors: Kuiper J, de Herder W, Brahim Y, van Velthuysen M, Brosens L,

Keywords: glucagon receptor mutation, mahvash disease, glucagon cell hyperplasia and neoplasia, pancreatic neuroendocrine tumour, MEN1,

#4237 Continuing Somatostatin Analogues Upon progression in Neuroendocrine tumor pAtients (SAUNA trial) – Study protocol for a bi-national, multi-center, open-label, randomised, pragmatic clinical trial

Introduction: Advanced gastroenteropancreatic neuroendocrine tumors (GEP NET) are treated with targeted therapy or peptide receptor radionuclide therapy (PRRT) (177Lu-DOTATATE) upon progression under somatostatin analogues (SSA). SSA are continued life-long in functional NET, but the benefit of SSA continuation in non-functional NET is unclear. Due to ongoing activity of the somatostatin receptor pathway in GEP NET progressing on SSA, we hypothesize an added efficacy of SSA during second-line therapy.

Conference:

Presenting Author: Chhajlani S

Authors: Chhajlani S, Kuiper J, Borbath I, Dercksen W, Deroose C,

Keywords: somatostatin analogues, SSA, everolimus, sunitinib, peptide receptor radionuclide therapy, PRRT, quality of life, QoL, gastroenteropancreatic neuroendocrine tumor, GEP NET,

#17 Diagnostic efficacy of the secretin stimulation test for the Zollinger-Ellison syndrome: an intra-individual comparison using different dosages in patients and controls

Introduction: The diagnosis of Zollinger-Ellison syndrome is suspected in patients with symptoms of gastric acid hypersecretion, i.e., peptic ulcer disease, malabsorption or diarrhea, or with elevated fasting serum gastrin levels. However, symptoms can be masked by the use of proton pump inhibitors and fasting serum gastrin values are not always conclusive. Therefore, the secretin stimulation test is advocated as the principal diagnostic tool to identify the Zollinger-Ellison syndrome.

Conference: 7th Annual ENETSConcerence (2010)

Presenting Author:

Authors: Kuiper P, Biemond I, Masclee A, Jansen J, Verspaget H,

Keywords: gastrinoma, secretin, gastrin, hypergastrinemia,

#16 Endoglin as indicator of metastatic neuroendocrine tumors of the pancreas

Introduction: Neuroendocrine tumors of the pancreas are rare, highly vascularized tumors. Endoglin, a Transforming Growth Factor-β co-receptor, is a marker for angiogenic endothelial cells. Angiogenesis is required for tumor progression and the development of metastases. Recently, endoglin expression was found to be a prognostic marker in pancreatic carcinomas. However, the role of endoglin in neuroendocrine pancreatic tumors has so far not been studied.

Conference: 7th Annual ENETSConcerence (2010)

Presenting Author:

Authors: Kuiper P, Verspaget H, de Jonge-Muller E, Hawinkels L, Biemond I,

Keywords: endoglin, pancreatic neuroendocrine tumor, angiogenesis, metastases,